Generalized epilepsy in hypothalamic hamartoma

Freeman, Jeremy L.; Harvey, A. Simon; Rosenfeld, Jeffrey V.; Wrennall, Jacquie; Bailey, Catherine; Berkovic, Samuel F.

doi:10.1212/01.wnl.0000049457.05670.7d

Cited by 146 publications

(147 citation statements)

References 20 publications

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“…Delalande and Fohlen 9 reported on 17 patients in 2003 who underwent surgery for HH (14 transsylvian and 9 endoscopic approaches). We subsequently reported our epilepsy findings, 13,17 endocrine findings, 14 and MR imaging findings. 12 There was a wide variation in the size and position of HH, and the attachments and relations of the HH were also described.…”

Section: The Royal Children's Hospital Experiencementioning

confidence: 93%

“…5,13,16 There are multiple possible routes by which seizures spread from the HH into adjacent and remote parts of the brain. One pathway is that the seizures spread directly through the mammillary body to the thalamus and beyond via the mammillothalamic tracts, 13 so that disconnection of the HH from the mammillary body has become one of the key goals of the disconnection surgery. Sessile HHs are attached to one or both mammillary bodies.…”

Section: The Development Of Surgery For Hhmentioning

confidence: 99%

“…These complex patients require an extensive preoperative workup, which has been described previously. 13,17,33 The first patient we operated on for HH and gelastic epilepsy at Royal Children's Hospital presented in 1996. The exophytic component of the lesion, attached to and extending below the hypothalamus, could be debulked via a subfrontal approach, but a significant component of the lesion was still present within the cavity of the third ventricle and was attached to the medial walls of the hypothalamus on the postoperative MR image.…”

Section: The Royal Children's Hospital Experiencementioning

confidence: 99%

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The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

FOC

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The prognosis for patients with hypothalamic hamartoma has improved dramatically over the last 20 years, for 3 main reasons. First, because of improved understanding of the anatomy and pathophysiology of these varied lesions. Second, due to advances in brain imaging and refinements in microsurgery, including the anterior transcallosal interforniceal approach, endoscopic, and skull-base approaches. And third, because of increasing experience with stereotactic radiosurgery, interstitial radiotherapy, and radiofrequency lesioning. Patients with hypothalamic hamartoma should be managed in comprehensive epilepsy centers where the treatments are individualized and concentrated in the hands of surgeons who can perform the full range of surgery, including approaches to the third ventricle. Total seizure-freedom rates of 52% to 66% have been achieved with surgery.

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Section: The Royal Children's Hospital Experiencementioning

confidence: 93%

Section: The Development Of Surgery For Hhmentioning

confidence: 99%

Section: The Royal Children's Hospital Experiencementioning

confidence: 99%

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The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

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“…The hypothesis of an intrinsic epileptogenesis of HH gained impetus after the stereo-EEG demonstration of ictal onsets originating from the hypothalamic lesion, 32 and was further corroborated by subsequent reports showing production of typical gelastic seizures by depth electrode stimulation, ictal SPECT studies demonstrating HH hyperperfusion, and interictal spike source analysis. 4,9,11,12,16,21,22,26,35,45 The epileptogenic activity of HHs has been recently demonstrated at the cellular level: detailed electrophysiological analysis of slices taken from surgical specimens showed the predominance of small GABAergic inhibitory neurons exhibiting an intrinsic "pacemaker-like" behavior. 54 The concept that surgical intervention should mainly target the HH was not easy to accept.…”

Section: Pathophysiology: Intrinsic Epileptogenesis Of Hhsmentioning

confidence: 99%

Radiosurgery for hypothalamic hamartomas

Romanelli

Muacevic²,

Striano

2008

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✓ Radiosurgery plays an important role in the treatment of refractory seizures induced by hypothalamic hamartomas (HHs). These lesions, deeply located and surrounded by a delicate vascular and neuronal network, are often associated with catastrophic epilepsy leading to progressive cognitive and behavioral deterioration. Surgical approaches include microsurgical resection, endoscopic resection or disconnection, radiofrequency lesioning, and interstitial brachytherapy. Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date.

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“…27,40 Disconnection by transventricular or transcallosal endoscopic approach has a good outcome and fewer complications than resective surgery. 1,8,14,24 There is also strong evidence that radiofrequency and Gamma Knife surgery may lead to complete seizure control with limited morbidity. 3,9,15,16,29,31,33,40,42 The Grenoble group hypothesizes that seizures arising from the HH spread to the cortex through the mamillothalamo-cingulate tract.…”

mentioning

confidence: 99%

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

Marras

Rizzi²,

Villani

et al. 2011

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Hypothalamic hamartomas (HHs) are developmental malformations associated with a range of neurological problems, including intractable seizures. There is increasing evidence of the epileptogenicity of the hamartoma and of the inhomogeneous distribution of the epileptic abnormalities within the malformation. The management strategy for treatment and results differ according to the insertion plane and the extension of the malformation into the hypothalamus. Cases characterized by extensive involvement of the hypothalamus are particularly challenging. The authors describe the case of a patient with drug-resistant epilepsy and a large hypothalamic hamartoma with an extensive area of attachment. The patient underwent implantation of 2 deep brain electrodes. The intraoperative recording showed a synchronous interictal epileptic discharge in the left temporal lobe and on the left side of the lesion. The patient was treated with chronic high-frequency stimulation. No side effects due to the stimulation were reported. At 18 months' follow-up, a reduction in complex partial seizure frequency was reported, but no significant reduction in overall seizure frequency was noticed (p = 0.14, t-test). The authors report on neurophysiological studies of the relationship between HH and epilepsy, and also discuss the literature on chronic high-frequency stimulation, including its rationale and the results of chronic stimulation of various targets for the treatment of drug-resistant epilepsy due to HH.

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Generalized epilepsy in hypothalamic hamartoma

Cited by 146 publications

References 20 publications

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Radiosurgery for hypothalamic hamartomas

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

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